Immunohistochemistry of paraffin-embedded Human liver damage using PEX5 Polyclonal Antibody at dilution of 1:100 (40x lens).
PEX5 Polyclonal Antibody
E-AB-61116
Product group Antibodies
Overview
- SupplierElabscience
- Product NamePEX5 Polyclonal Antibody
- Delivery Days Customer12
- CertificationResearch Use Only
- Scientific DescriptionThe product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.
- UNSPSC12352203